[Clinical presentation and diagnosis of epileptic auras].

To define clinical presentations of visual auras and to reveal their clinical, encephalographic and neuroimaging correlates, we examined 23 patients, aged from 5 to 25 years (mean 14±6 years), with focal forms of epilepsy. Patients had visual auras regardless of the etiology of epilepsy which developed immediately before epileptic seizures or were isolated. Patients had simple or complex visual hallucinations, the former occurring more frequently, visual illusions and ictal amaurosis. Positive visual phenomena were noted more frequently than negative ones. In most of the patients, visual hallucinations were associated with the pathological activity in cortical occipital regions of the brain and, in some cases, in temporal and parietal regions. The different pathologies (developmental defects, post-ischemic, atrophic and other disturbances) identified by MRI were found in a half of patients.

[Efficacy and safety of the mono- and combined therapy with oxcarbazepine in adult patients].

The trial included 302 patients with focal and generalized forms of epilepsy, aged from 18 to 73 years (mean age 33.23+/-12.73 years). Oxcarbazepine (trileptal) was prescribed in accordance to recommendations and titration schemes (continuous and single-stage) conventional in Russia. Trileptal was prescribed as the start antiepileptic therapy to 46.1% of patients. In other patients, antiepileptic drugs used previously were withdrawn mainly to their low clinical efficacy and poor tolerability. The duration of the study was 12 weeks. The efficacy was assessed by the number of patients with seizures reductions of 50%. The positive therapeutic response was found in 93.2% of patients who completed the trial, and the complete reduction of seizures was achieved in 34.9%. There were also positive changes in the seizure duration, frequency of consciousness change and other post seizure phenomena. The low frequency and intensity of adverse effects during the treatment with trileptal was observed: there were 93 adverse effects in 60 patients included in the trial; 3 (3.2%) of these effects were considered as severe (dizziness in one patient, sleepiness in two patients). The adverse effects disappeared without any additional treatment.

[Epilepsy with electrical status epilepticus during slow sleep: diagnostic criteria and approaches to therapy].

Fourteen patients, aged from 5 to 14 years, with syndrome of electrical status epilepticus during slow sleep (ESESS) have been studied. The absence of epileptic attacks was observed in 21.5% of patients and diagnosis was established by a combination of continuous diffuse epileptiform activity with marked cognitive disturbances. In 78.5% patients, epileptic attacks presented as follows: pharyngeal and oral, hemicephalgia (100% patients with attacks), hemiclonic (18%), atypical absences (27%), negative myoclonus (18%), automotor (18%), focal adverse with vomiting (18%), secondary generalized (36%). Drugs of choice were valproate (depakine) and topamax in patients with attacks; suxilep and frisium in the absence of attacks and in case of continuous diffuse epileptiform activity on the sleep EEG. Two variants have been singled out by the character of ESESS syndrome. The first one, a "symptom variant", was featured by mostly hemiclonic, secondary generalized and automotor epileptic attacks, presence of continuous regional or lateral, less frequent diffuse epileptiform activity, detected by the local structural lesions in MRI, marked cognitive disturbances persisting after stopping of the attacks. The second ("idiopathic") variant is characterized by normal development of children before attack manifestation, appearance of mostly "rolandic" attacks, atypical absences and negative myoclonus, presence of exclusively continuous diffuse epileptiform activity in the phase of slow sleep, standard MRI results (moderate cortical subatrophy in single cases), cessation or substantial decrease of cognitive disturbances after attack stopping. It is emphasized that prognosis of ESESS syndrome should be considered separately with regard to attacks and cognitive disturbances. A prognosis for attacks is always favorable. Cognitive disturbances despite the therapy can persist for a long time.

[Topamax in monotherapy of epilepsy]. / Topamaks pri monoterapii épilepsii.

Thirty-three patients, aged 3-29 years, with the following epileptic types: symptomatic forehead (15), symptomatic temporal (6), symptomatic occipital (2), juvenile myoclonic, in combination with eyelid myoclonus syndrome with absences, (5), epilepsy with isolated generalized seizures (3) and rolandic epilepsy (2), were treated with topamax. A medication dose was 50-200 mg per day in children younger 12 years and 100-550 mg per day in those older 12 years and in adults. The results obtained suggest the high efficacy and well tolerability of topamax in monotherapy of epilepsy. Therapeutic effect was achieved in 28 out of 33 patients (84.8%), i.e. seizures stopping--in 18 patients (54.5%). Monotherapy was mostly effective in symptomatic forehead epilepsy: seizures stopped in 53.3% patients and a frequency of seizures reduced in 33.3%. Side-effects were detected only in 18% cases, they were mostly transient and resulted in treatment withdrawal in 6% patients only.

[Electroclinical characteristics of Landau-Kleffner syndrome]. / Elektroklinicheskaia kharakteristika sindroma Landau-Kleffnera.

The study aimed at investigating epileptic attack's semiology and other electroclinical characteristics in Landau-Kleffner syndrome as well as therapeutic efficacy. Six patients with Landau--Kleffner syndrome, 5 boys, 1 girl, aged 6-10 years, mean age 7.5 years, have been analyzed. Epileptic attacks were observed only in 3 patients with debut at the age of 2.5-6 years (mean 4 years). In 50% of the cases, the attacks were not detected. The types of epileptic attacks were specified as follows: atypical absences--3 patients; pharyngo-oral--2; secondary generalized--2; atonic falls--1; hemiconvulsive--1. In routine EEG study, regional epileptiform activity was observed in all the patients, being localized in central temporal (3 cases), posterior temporal (1) and fronto-temporal (1) leads. In 3 cases (50%), diffusive epiactivity was also detected. During sleep, emergence of prolonged diffuse epiactivity was revealed for all the patients, with appearance of electrical status epilepticus during slow sleep in 50%. Valproates in dosage 30-60 mg/kg daily were highly effective for stopping epileptic seizures. Significant improvement of speech functions was observed only if antiepileptics sulthiame (ospolot) or clobasam (frizium) were used in addition to basic therapy. Clobasam in dosage 0.5-0.75 mg/kg daily was the most efficient in blockade of EEG diffuse epileptiform activity, reduction of aphasia symptoms and behavior improvement.

[Diagnostic differentiation between two syndromes of progressive myoclonus epilepsy]. / Differentsial'nyi diagnoz dvukh sindromov progressiruiushchei mioklonus-épilepsii.

The study aimed to differentiate, according to clinical and electroencephalographical criteria, between the most frequent types of myoclonus epilepsy--Unverricht-Lundborg disease (ULD) and Lafora disease (LD). Two patients with ULD and two with LD, aged 13-16 years, have been examined. In all cases, the diagnosis of myoclonus epilepsy has been verified by using molecular genetic analysis. The differential diagnostic criteria have been ascertained for ULD and LD: the earlier age-of-onset in ULD comparing to LD (8.8 and 12.5 years, respectively); tonic clonic paroxysms in ULD and partial ones with visual aura in LD; a presence of nonepileptical subcortical myoclonus, according to EEG data (visual monitoring); negative myoclonus emerging in manifested stage of LD; appearing of regional epileptiform activity for posterior regions on EEG in LD; more rapid progressiveness of extra pyramidal disturbances with organic dementia outcome in myoclonus LD.

[Diagnostic criteria of atypical benign partial epilepsy syndrome in childhood]. / Diagnosticheskie kriterii sindroma atipichnoi dobrokachestvennoi partsial'noi épilepsii detskogo vozrasta.

The paper presents an analysis of clinical-neuropsychological peculiarities of the syndrome of atypical benign partial childhood epilepsy (pseudolennox syndrome) of 6 patients (3 boys, 3 girls). An age of the onset of the disease was between 1.5-4 years. There was polymorphism of paroxysms, their high frequency with an obligate presence of hemifacial fits and atypical absences. Night generalized tonic-clonic attacks and the falling attacks were found in 67% of the patients. Spectrum of the neurological disorders included disorders of speech and a slight cerebella symptomatology. Regional "rolandic" activity and diffuse epileptiformed disorders, increasing into a phase of a slow sleep, were registered by EEG. Resistance to anticonvulsive therapy was revealed. The authors had demonstrated a nosologic independence of pseudolennox syndrome and had considered worth while to pick it out in a group of cryptogenic partial forms of epilepsy together with the epileptic aphasia of Landu-Kleffner and an epilepsy with the continuous peak-waves during the slow sleep.

[Benign myoclonic epilepsy in infancy]. / Dobrokachestvennaia mioklonicheskaia épilepsiia mladenchestva.

Benign myoclonic epilepsy in infancy (NMEI) is one of rare epileptic syndromes. 5 patients (all female sex) aged 4-16 years were observed. NMEI debuted at the age from 7 months till 2.5 years (mean age 1.3 years). Pathology of pregnancy and labor, disorders in both psychomotor development and genetic predisposition were not found. In all the cases the disease began with typical transitory repeated myoclonic paroxysms of different intensity and frequency, without loss of consciousness and with primary involvement of the muscles of the neck and the upper extremities. Most patients had muscular hypotension, mild coordinatory disorders, delayed psycho-speech development, mental retardation, EEG signs of generalized epileptic activity. Valproates, suxilep, clonazepam and lamotrigin (lamiktal) were used for treatment. The most pronounced effect was achieved using either monotherapy with valproates (depakin) or a combination depakin + lamiktal. A stable clinical-encephalographic remission was achieved in all the patients, but during puberty in 2 patients (15 and 16 years old) rare generalized convulsive fits debuted. High frequency of intellectual-mnestic disorders were found even after a complete remission. So benign definition concerns only a course of the fits, but not NMEI prognosis.